Issue 7/2021
Content (40 Articles)
Harnessing Type I IFN Immunity Against SARS-CoV-2 with Early Administration of IFN-β
Donald C. Vinh, Laurent Abel, Paul Bastard, Matthew P. Cheng, Antonio Condino-Neto, Peter K. Gregersen, Filomeen Haerynck, Maria-Pia Cicalese, David Hagin, Pere Soler-Palacín, Anna M. Planas, Aurora Pujol, Luigi D. Notarangelo, Qian Zhang, Helen C. Su, Jean-Laurent Casanova, Isabelle Meyts, Alessandro Aiuti, Lisa M. Arkin, Alexandre Bolze, Samya Charkravorty, John Christodoulou, Roger Colobran, Beth A. Drolet, Jacques Fellay, Pr Antoine Froidure, Jean William Pape, Rabih Halwani, Trine H. Mogensen, Giuseppe Novelli, Igor B. Resnick, Anna Sediva, Ivan Tancevski, Stuart Turvey
Another Exciting Data—HCT Successfully Cured Patients with DADA2
Motoi Yamashita, Tomohiro Morio
Human Disease Phenotypes Associated with Loss and Gain of Function Mutations in STAT2: Viral Susceptibility and Type I Interferonopathy
Christopher James Arthur Duncan, Sophie Hambleton
Atypical Inflammatory Syndrome Triggered by SARS-CoV-2 in Infants with Down Syndrome
Louise Malle, Paul Bastard, Andrea Martin-Nalda, Taya Carpenter, Douglas Bush, Roosheel Patel, Roger Colobran, Pere Soler-Palacin, Jean-Laurent Casanova, Melissa Gans, Jacques G. Rivière, Dusan Bogunovic
COVID-19 in the Context of Inborn Errors of Immunity: a Case Series of 31 Patients from Mexico
Lina M. Castano-Jaramillo, Marco Antonio Yamazaki-Nakashimada, Patricia M. O’Farrill-Romanillos, David Muzquiz Zermeño, Selma C. Scheffler Mendoza, Edna Venegas Montoya, Jorge Alberto García Campos, Luz María Sánchez-Sánchez, Luisa B. Gámez González, Jesús Moisés Ramírez López, Juan Carlos Bustamante Ogando, Estefanía Vásquez-Echeverri, Edgar Alejandro Medina Torres, Gabriela Lopez-Herrera, Lizbeth Blancas Galicia, Laura Berrón Ruiz, Aidé Tamara Staines-Boone, Sara Elva Espinosa-Padilla, Nora Hilda Segura Mendez, Saul O. Lugo Reyes
Outcome of SARS-CoV-2 Infection in 121 Patients with Inborn Errors of Immunity: A Cross-Sectional Study
Ekaterini Simões Goudouris, Fernanda Pinto-Mariz, Leonardo Oliveira Mendonça, Carolina Sanchez Aranda, Rafaela Rolla Guimarães, Cristina Kokron, Myrthes Toledo Barros, Flávia Anísio, Maria Luiza Oliva Alonso, Fernanda Marcelino, Solange Oliveira Rodrigues Valle, Sergio Dortas Junior, Irma Douglas Paes Barreto, Janáira Fernandes Severo Ferreira, Pérsio Roxo-Junior, Almerinda Maria do Rego Silva, Fernanda Lugão Campinhos, Carmem Bonfim, Gisele Loth, Juliana Folloni Fernandes, Julia Lopes Garcia, Albertina Capelo, Olga Akiko Takano, Maria Isabel Valdomir Nadaf, Eliana C. Toledo, Luciana Araújo Oliveira Cunha, Regina Sumiko Watanabe Di Gesu, Laire Schidlowski, Priscila Fillipo, Daniélli C. Bichuetti-Silva, Gustavo Soldateli, Natasha Rebouças Ferraroni, Ellen de Oliveira Dantas, Simone Pestana, Eli Mansour, Raisa Gusso Ulaf, Carolina Prando, Antonio Condino-Neto, Anete Sevciovic Grumach
Antibody Response to SARS-CoV-2 is Associated with Long-term Clinical Outcome in Patients with COVID-19: a Longitudinal Study
Javier García-Abellán, Sergio Padilla, Marta Fernández-González, José A. García, Vanesa Agulló, María Andreo, Sandra Ruiz, Antonio Galiana, Félix Gutiérrez, Mar Masiá
SARS-CoV-2 infection in a pediatrics STAT1 GOF patient under Ruxolitinib therapy-a matter of balance?
Paloma Guisado Hernández, Pilar Blanco Lobo, Isabel Villaoslada, Beatriz de Felipe, José M. Lucena, Guillermo Martín Gutierrez, María José Castro, Alicia Gutiérrez Valencia, María Isabel Sánchez Codez, Mirella Gaboli, Olaf Neth, Peter Olbrich
SARS-CoV-2–Related Acute Respiratory Distress Syndrome Uncovers a Patient with Severe Combined Immunodeficiency Disease
Bandar Al-Saud, Khaled M. Hazzazi, Reem Mohammed, Alaa Al Najjar, Tariq Al Hazmi, Dorota Monies, Fowzan S. Alkuraya
SARS-CoV-2 infection inducing severe flare up of Deficiency of Interleukin Thirty-six (IL-36) Receptor Antagonist (DITRA) resulting from a mutation invalidating the activating cleavage site of the IL-36 receptor antagonist
Alizée Bozonnat, Florence Assan, Jérôme LeGoff, Emmanuelle Bourrat, Hervé Bachelez
COVID-19 in Patients with Primary Immunodeficiency
Saliha Esenboga, Melike Ocak, Ayşegul Akarsu, Hacer Neslihan Bildik, Deniz Cagdas, Arzu Topeli Iskit, Ilhan Tezcan
Containment of Local COVID-19 Outbreak Among Hematopoietic Stem Cell Transplant Recipients and Healthcare Workers in a Pediatric Stem Cell Unit
Monia Ouederni, Samia Rekaya, Oussema Bouabdallah, Ilhem Ben Fradj, Ridha Kouki, Yosr Chebbi, Sahar Ben Ammar, Takwa Lamouchi, Asma Lachiheb, Nessrine Zekri, Siwar Laajili, Ikram Zaiter, Agnes Hamzaoui, Mohamed Bejaoui, Fethi Mellouli, Wafa Achour, Monia Ben Khaled
Safety of COVID-19 Vaccination in Immune-Deficient Patients Receiving Supplemental Immunoglobulin Therapies
Jacqueline D. Squire, Avni Y. Joshi
Neutralizing Anti-interferon-γ Autoantibodies: an Ameliorating Factor in COVID-19 Infection?
Mark Kacar, Paulina Cortes-Acevedo, Vinesh Patel, Clive Carter, Pamela Hughes, Hugh P. McGann, Effrossyni Gkrania-Klotsas, Helen E. Baxendale, Gabriela Barcenas-Morales, Rainer Doffinger, Sinisa Savic
Lineage-Specific Chimerism and Outcome After Hematopoietic Stem Cell Transplantation for DOCK8 Deficiency
Johannes Raedler, Thomas Magg, Meino Rohlfs, Christoph Klein, Tanja Vallée, Fabian Hauck, Michael H. Albert
Pulmonary Hypertension in Patients with Common Variable Immunodeficiency
Pierre Thoré, Xavier Jaïs, Laurent Savale, Peter Dorfmuller, Athénaïs Boucly, Matthieu Devilder, Olivier Meyrignac, Jérémie Pichon, Julie Mankikian, Marianne Riou, Emmanuel Boiffard, Clément Boissin, Pascal De Groote, Céline Chabanne, Frédéric Gagnadoux, Anne Bergeron, Nicolas Noel, Olivier Sitbon, Marc Humbert, David Montani
Single-Center Study of 72 Patients with Severe Combined Immunodeficiency: Clinical and Laboratory Features and Outcomes
Ozlem Bayram, Sule Haskologlu, Deniz Bayrakoğlu, Sevgi Kostel Bal, Candan Islamoglu, Funda Erol Cipe, Tanil Kendirli, Nazmiye Kursun, Sukru Nail Guner, Alisan Yildiran, Gunseli Bozdogan, Mutlu Yuksek, Ismail Reisli, Klara Dalva, Caner Aytekin, Kaan Boztug, Figen Dogu, Aydan Ikinciogullari
Haematopoietic Stem Cell Transplant for Norovirus-Induced Intestinal Failure in X-linked Agammaglobulinemia
Ben M. J. Shillitoe, Mark Ponsford, Mary A. Slatter, Jennifer Evans, Siske Struik, Mike Cosgrove, Iolo Doull, Stephen Jolles, Andrew R. Gennery
T Cell-Epstein-Barr Virus–Associated Hemophagocytic Lymphohistiocytosis (HLH) Occurs in Non-Asians and Is Associated with a T Cell Activation State that Is Comparable to Primary HLH
Oded Shamriz, Deepak Kumar, Jenny Shim, Michael Briones, Maa-Ohui Quarmyne, Satheesh Chonat, Laura Lucas, Holly Edington, Michael H. White, Advay Mahajan, Sunita Park, Shanmuganathan Chandrakasan
Normal IgH Repertoire Diversity in an Infant with ADA Deficiency After Gene Therapy
Carolyn H. Baloh, Samiksha A. Borkar, Kai-Fen Chang, Jiqiang Yao, Michael S. Hershfield, Suhag H. Parikh, Donald B. Kohn, Maureen M. Goodenow, John W. Sleasman, Li Yin
Elevated Expression Levels of Lung Complement Anaphylatoxin, Neutrophil Chemoattractant Chemokine IL-8, and RANTES in MERS-CoV-Infected Patients: Predictive Biomarkers for Disease Severity and Mortality
Maaweya E. Hamed, Asif Naeem, Haitham Alkadi, Aref A. Alamri, Ahmad S. AlYami, Abdullah AlJuryyan, Wael Alturaiki, Mushira Enani, Samia T. Al-Shouli, Abdullah M. Assiri, Bandar Alosaimi
Chronically Activated T-cells Retain Their Inflammatory Properties in Common Variable Immunodeficiency
Roos-Marijn Berbers, M. Marlot van der Wal, Joris M. van Montfrans, Pauline M. Ellerbroek, Virgil A. S. H. Dalm, P. Martin van Hagen, Helen L. Leavis, Femke van Wijk
Hematopoietic Cell Transplantation Cures Adenosine Deaminase 2 Deficiency: Report on 30 Patients
Hasan Hashem, Giorgia Bucciol, Seza Ozen, Sule Unal, Ikbal Ok Bozkaya, Nurten Akarsu, Mervi Taskinen, Minna Koskenvuo, Janna Saarela, Dimana Dimitrova, Dennis D. Hickstein, Amy P. Hsu, Steven M. Holland, Robert Krance, Ghadir Sasa, Ashish R. Kumar, Ingo Müller, Monica Abreu de Sousa, Selket Delafontaine, Leen Moens, Florian Babor, Federica Barzaghi, Maria Pia Cicalese, Robbert Bredius, Joris van Montfrans, Valentina Baretta, Simone Cesaro, Polina Stepensky, Neven Benedicte, Despina Moshous, Guillaume Le Guenno, David Boutboul, Jignesh Dalal, Joel P. Brooks, Elif Dokmeci, Jasmeen Dara, Carrie L. Lucas, Sophie Hambleton, Keith Wilson, Stephen Jolles, Yener Koc, Tayfun Güngör, Caroline Schnider, Fabio Candotti, Sandra Steinmann, Ansgar Schulz, Chip Chambers, Michael Hershfield, Amanda Ombrello, Jennifer A. Kanakry, Isabelle Meyts
A Case of VEXAS Syndrome Complicated by Hemophagocytic Lymphohistiocytosis
Alice Grey, Pak Leng Cheong, Frederick J. Lee, Edward Abadir, James Favaloro, Shihong Yang, Stephen Adelstein
A Case Report of SAVI Mimicking Early-Onset ANCA Vasculitis
Elisa Ochfeld, Megan L. Curran, Sergio E. Chiarella, Kaveh Ardalan, Amer Khojah
Hemophagocytic Lymphohistiocytosis in Activated PI3K Delta Syndrome: an Illustrative Case Report
Zijun Zhou, Timo Zondag, Maud Hermans, P. Martin van Hagen, Jan A. M. van Laar
COPA Syndrome (Ala239Pro) Presenting with Isolated Follicular Bronchiolitis in Early Childhood: Case Report
Pamela Psarianos, Jennifer Yin Yee Kwan, Sharon Dell, Wallace B. Wee, Katrina Rey-McIntyre, Haiying Chen, Dilan Dissanayake, Ronald M. Laxer, Anthony Shum, Fei-Fei Liu, Kenneth W. Yip
Curative Treatment of POMP-Related Autoinflammation and Immune Dysregulation (PRAID) by Hematopoietic Stem Cell Transplantation
Andrea Meinhardt, Paula C. Ramos, R. Jürgen Dohmen, Nadja Lucas, Min Ae Lee-Kirsch, Benjamin Becker, Jan de Laffolie, Tomás Cunha, Tim Niehues, Ulrich Salzer, Ayami Yoshimi, Miriam Erlacher, Anke M. J. Peters, Stephan Ehl, Brigitte Strahm, Carsten Speckmann
Hematopoietic Stem Cell Transplantation Successfully Treats CD40LG Duplication
Di Sun, Carole Le Coz, Nancy Bunin, Neil Romberg
ALPK1 Gene Mutations Drive Autoinflammation with Ectodermal Dysplasia and Progressive Vision Loss
Yvan Jamilloux, Thibaud Mathis, Olivier Grunewald, Hélène Dollfuss, Thomas Henry, Pascal Sève, Isabelle Meunier
Exploration of Potential Immunodeficiency Unveils Hennekam Lymphangiectasia-Lymphedema Syndrome
Sara Kaut, Giorgia Bucciol, Leen Moens, Isabelle Meyts
Listeria Monocytogenes Meningoencephalitis Due to IRAK4 Deficiency
Ding Liu, Ru Chen, Longfeng Ran, Ruxu Zhang, Han Chen
Alternative Approach to Varicella Zoster Virus Prevention in a Child with Lymphopenia
Manuela Cortesi, Raffaele Badolato
Myelodysplastic Syndrome in a Patient with IPEX Syndrome
Daisuke Toyama, Akihiro Hoshino, Kento Inoue, Takahiro Kamiya, Hirokazu Kanegane, Shohei Yamamoto
Achromobacter xylosoxidans Pneumonia in a Young Child with Chronic Granulomatous Disease—a Case-Based Review
Pratap Kumar Patra, Aaqib Zaffar Banday, Rohit Sadanand, Bhawna Sharma, Archana Angrup, Pandiarajan Vignesh, Amit Rawat
Deficiency of Adenosine Deaminase 2—a Monogenic Cause of Wunderlich Syndrome
Pratap Kumar Patra, Sanjib Kumar Mondal, Manphool Singhal, Rajni Kumrah, Ankur Kumar Jindal, Michael Hershfield, Surjit Singh
Macrophage Activation Syndrome in a Patient with Prolidase Deficiency: a Rare Genetic Disorder Associated with Elevated IgE and Lupus-Like Syndrome
Aakash Chandran Chidambaram, Santhosh Kumar Thangaraju, Sanjana Sarangarajan, Kaushik Maulik, Jaikumar Govindaswamy Ramamoorthy, Dhandapany Gunasekaran
BCG Vaccine–Associated Complications in Patients with PTEN Hamartoma Tumor Syndrome
Henry Taylor, Dominik Aschenbrenner, Mike Thomson, Hannah Chen, Holm H. Uhlig
Killing Two Birds with One Stone: the Therapeutic Role of Ibrutinib in Schnitzler Syndrome
Chen Wang
Correction to: Clinical Manifestations, Mutational Analysis, and Immunological Phenotype in Patients with RAG1/2 Mutations: First Cases Series from Mexico and Description of Two Novel Mutations
Saul Oswaldo Lugo-Reyes, Nina Pastor, Edith González-Serrano, Marco Antonio Yamazaki-Nakashimada, Selma Scheffler-Mendoza, Laura Berron-Ruiz, Guillermo Wakida, Maria Enriqueta Nuñez-Nuñez, Ana Paola Macias-Robles, Aide Tamara Staines-Boone, Edna Venegas-Montoya, Carmen Alaez-Verson, Carolina Molina-Garay, Luis Leonardo Flores-Lagunes, Karol Carrillo-Sanchez, Julie Niemela, Sergio D. Rosenzweig, Paul Gaytan, Jorge A. Yañez, Ivan Martinez-Duncker, Luigi D. Notarangelo, Sara Espinosa-Padilla, Mario Ernesto Cruz-Munoz